Sickle cell disease: Recommended screening and interventions

Screen annually for albuminuria: spot urine test to estimate protein-to-creatinine ratio
If micro- or macroalbuminuria is present: 24-hour urine test
If protein excretion rate > 300 mg/24 hours, refer to a nephrologist
Consider angiotensin-converting enzyme inhibitor therapy
Assess for respiratory problems
Pulmonary function testing
If findings suggest pulmonary hypertension, refer for cardiology evaluation
Screen; treat to ≤ 130/80 mm Hg a
Refer to an ophthalmologist for a dilated eye examinationb; rescreen in 1–2 years if normal
Refer to a retinal specialist for suspected retinopathy
Screening limited to children
Blood transfusion: simple or exchange
Leg ulcers
Inspect lower extremities for active and healed ulcers
Treat with debridement, wet-to-dry dressings, topical agents
Chronic recalcitrant deep leg ulcers: evaluate for osteomyelitis, consult wound care specialist
Reproductive counseling
Reproductive life plan
Refer partners for hemoglobinopathy status testing if status is unknown
Test women anticipating pregnancy for red blood cell alloantibodies
Discuss contraception choices with no restrictions for use in sickle cell disease: progestin-only contraceptives, barrier methods; reinforce the need for barrier methods for patients on hydroxyurea
Avascular necrosis
Elicit from history and physical examination
Confirm with radiography and magnetic resonance imaging
Refer for physical therapy, orthopedic clinic